Introduction: Desmoid tumor of bone is a rare benign tumor. It is reported commonly in mandibular and meta-diaphyseal region of long bones. We report involvement of unusual site in ilio-acetabular region with breach in articular cartilage.

Case Report: A 40 year old female presented with pain in the left hip. Radiologically, a lytic lesion at ilio-acetabular region was seen. Intra-operatively breach in acetabular roof was seen which was missed in the scan. Curettage and defect reconstruction was done. Histopathology reported as desmoid tumor. 20 months post-operatively patient was symptom free.

Conclusion: Desmoid tumor is a rare bone tumor. This case report emphasizes about the rarity of the lesion in this location and the rare chances of breach in articular cartilage of the joint. The chances of recurrences are high with intralesional curettage.

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